Sudden Death in a Patient with Pulmonary Veno-occlusive Disease (PVOD) and Severe Pulmonary Hypertension

Intern Med. 2017;56(15):2025-2031. doi: 10.2169/internalmedicine.56.7869. Epub 2017 Aug 1.

Abstract

A 58-year-old woman was referred to our hospital with a chief complaint of exertional dyspnea. Bronchoscopy failed to establish a diagnosis, and the patient subsequently died suddenly due to respiratory insufficiency because of advanced pulmonary hypertension (PH). The pathological diagnosis at autopsy was pulmonary veno-occlusive disease (PVOD). PVOD is difficult to diagnose antemortem and has a poor prognosis. Lung transplantation is the only curative treatment for PVOD.

Keywords: exertional dyspnea; pulmonary hypertension; pulmonary veno-occlusive disease; sudden death.

Publication types

  • Case Reports

MeSH terms

  • Autopsy
  • Bronchoscopy
  • Death, Sudden / etiology*
  • Death, Sudden / pathology
  • Dyspnea / etiology
  • Female
  • Humans
  • Hypertension, Pulmonary / etiology*
  • Lung / pathology
  • Lung Transplantation
  • Middle Aged
  • Pulmonary Veno-Occlusive Disease / complications*
  • Pulmonary Veno-Occlusive Disease / diagnostic imaging
  • Pulmonary Veno-Occlusive Disease / pathology
  • Radiography
  • Respiratory Insufficiency / etiology
  • Tomography, X-Ray Computed